LearningRadiology - Lymphangiomyomatosis

Lymphangiomyomatosis and

Tuberous Sclerosis

Similar in pathology and x-ray appearance
Widespread proliferation of smooth muscle in pleura, alveolar septa, bronchi, pulmonary vessels and lymphatics as well as lymph nodes, especially in posterior mediastinum and retroperitoneum
Focal emphysema develops as result of narrowing of airways
Thoracic duct may be obliterated
Produce multiple small cysts with a hamartomatous proliferation of smooth muscle in their walls

Characteristic x-ray triad of:

Progressive, diffuse interstitial disease
Recurrent chylous effusions and sometimes chylous ascites
Recurrent pneumothorax

Tuberous sclerosis is inherited as a dominant with variable penetrance:

·          Mental defects
·       Epilepsy
·       Retinal phacoma
·       Angiomyolipomas of the kidneys
·       Rhabdomyomas of the heart
·       Intracranial calcifications
·       Sclerotic skull lesions
·       Adenoma sebaceum
·       Subungual fibromas

· Pulmonary lymphangiomyomatosis (syn: pulmonary myomatosis)

· Exclusively in females ages 17-47 years

· Rare

X-ray

Identical in both tuberous sclerosis and lymphangiomyomatosis and indistinguishable from pulmonary fibrosis except for decreased lung volume in fibrosis and increased lung volume in the others
Generalized, coarse reticulonodular changes
Unilateral or bilateral pleural effusions which are usually large and recurrent
Spontaneous pneumothorax is common
Clinically
Shortness of breath
Hemoptysis